Lung Disease Flashcards
what is interstitial lung disease
any disease process affecting lung interstitium (tissue and space around air sacs i.e. alveoli, terminal bronchi)
what does ILD interfere with
gas transfer
what type of lung pattern does interstitial lung disease produce
restrictive
what are the symptoms of ILD
breathlessness dry cough
what are the three types of interstitial lung disease
acute, episodic, chronic
describe the three main factors of chronic lung disease
part of systemic disease, exposure to agent, idiopathic
describe sarcoidosis- immunologically, it causes and the systems it invloves
granulomatus; type 4 hypersensitivity, non caseating granulomas, multisystem involvement; lungs, lymph nodes, joints, liver, skin. idiopathic
what are the two types of sarcoidosis
acute, chronic; lung infiltrates- alveolitis
what are the differential diagnosis of sarcoidosis
TB, lymphoma, carcinoma, fungal infection
how is sarcoidosis diagnosed
CXR and CT scan- if not blood test
how is acute sarcoidosis treated
self limiting, steroids if vital organ affected
how is chronic sarcoidosis treated
with oral steroids- sarcoidosis very sensitive to steroids, immunosupression
what needs to happen after the treatment of sarcoidosis
monitor chest x-ray and pulmonary function for several year- often relapses
what are the symptoms of sarcoidosis
small patches of red and swollen skin, usually affects lungs and skin
what eye condition can be caused by sarcoidosis what how is it treated
uveitis, steroid drops
describe the histology of sarcoidosis
non-caseating, multinucleated giant cells
what is extrinsic allergic alveolitis a form of
hypersensitivity pneumonitis, type 3 hypersensitivity
what are the two forms of extrinsic allergic alveolitis
acute and chronic
what are the symptoms of acute E.A.A
cough, breathless, fever, myalgia
how is acute E.A.A treated
oxygen, steroid and antigen avoidance
describe chronic E.A.A
repeated low odse antigen exposure over time
what are the symptoms of chronic E.A.A
progressive breathlessness and cough, maybe crackles
what will an x ray show in chronic E.A.A
CXR show pulmonary fibrosis, commonly in upper lung
what will a PFT show in chronic E.A.A
restrictive defect ( low FEV1 and FVC, high/normal ratio, low gas transfer)
how is chronic E.A.A treated
remove antigen exposure, oral steroids
what might a CXR show in sarcoidosis
bilateral hilar lymphadenopathy or enlargement and lung infiltrates
what is the most common form of ILD
idiopathic pulmonary firbrosis
is idiopathic pulmonary fibrosis an inflammatory condition
no
what is thought to cause IPF
imbalance of fibrotic repair system, related to gastric reflux, more common in smokers
what are the secondary causes of IPF
rheomatoid, asbestos, drugs
what is the clinical presentation of IPF
progressive breathlessness over many years, dry cough, clubbing, bilateral inspiratory crackles,
how does IPF show on a PFT
restrictive defect, low gas transfer
how does IPF show on a CXR
bilateral infiltrates, interstitial scarring
how does IPF show on a CT scan
reticulonodular fibrotic shadowing, worse at bases and periphery. traction bronchiectasis, honey-combing cystic changes
how is IPF diagnosed
history (ask about occupation), examination and radiology tests
what needs to be done is presentation is atypical
lung biopsy
describe the pathology of IPF
interstitial pneumonia pattern, heterogeneous fibrosis in alveolar walls causing honey-combing
how is IPF treated
anti firbrotic treatments slow progress do not reverse- pirfenidone and nintedanib
when are steroids used to treat IPF
never, only work with inflammatory ILD
what is coal workers pneumoconiosis
simple pneumoconiosis (restrictive lung disease cause by inhalation of dust)
what is complicated pneumoconiosis
progressive massive firbosis
what causes chronic bronchitis
coal dust + smoking
what is chronic bronchitis
inflamed bronchial tubes with excessive mucous production
what is caplans syndrome and what causes it
rheumatoid pneumoconiosis- when a patient with rheumatoid arthritis gets exposed to coal dust and gets cavitating nodules in lung
what is rheumatism
inflammation and pain in the joint
what does an CXR of simple coal workers pneumoconiosis
abnormality- miliary dots, shadows
what symptoms does simple (coal workers) pneumoconiosis present
none- risk of developing complication later
what causes silicosis
15-20 years exposure to quartz (mining, glass workers, boiler workers, foundry workers)
what does a CXR of silicosis show
eggshell calcification of hilar nodes
how is chronic silicosis characterised
restrictive pattern, pulmonary fibrosis
what does a CXR of a simple pneumconiosis (silicosis) show
miliary nodular appearance
describe progressive massive fibrosis
balls of fibrous tissue that grow and engulf the lungs
what does exposure to barium cause
baritosis
describe asbestosis
pulmonary fibrosis due to heavy, prolonged exposure to asbestos
what are the 4 pleural diseases relating to asbestos
benign pleural plaques (asymptomatic)
acute asbestos pleuritis (fever, pain, bloody pleural effusion), pleural effusion and diffuse pleural thickening (restrictive impairment), malignant mesothelioma (incurable pleural cancer)
what are the presenting symptoms of malignant mesothelioma
chest pain and pleural effusion
what is pleural effusion
build up of fluid in pleural space
what does asbestos multiply the risk of in smokers
bronchial carcinoma
how do fibrous cause fibrosis
cause abnormal behaviour in macrophages and fibres laid down
what is pulmonary interstitium
alveolar lining cells (type 1 and 2), thin elastic-rich connective component containing capillary blood vessels
describe the effect of early stage alveolitis on the alveolar walls
injury with inflammatory cell infiltration
what is acute ILD exemplified by
adult respiratory distress syndrome
what is late stage interstitial lung disease characterised by
fibrosis
what do patients with ILD present with
clinical effects due to hypoxia- respiratory failure and cardiac failure. Also seen on radiology
what can cause interstitial lung disease
environment (minerals, drugs, radiation- HYPERSENSITIVITY), unknown- idiopathic (connective tissue diseases, idiopathic pulmonary fibrosis)
what is idiopathic pulmonary fibrosis
progressive fibrotic disease
what types of biopsy used to diagnose interstitial lung disease
transbronchial, thoracoscopic more invasive but more reliable
what conditions are associated with chronic interstitial lung disease
idiopathic pulmonary fibrosis, sarcoidosis, extrinsic allergic alveolitis (hypersensitivity pneumonitis), pneumoconiosis, connective tissue diseases
what symptoms are associated idiopathic pulmonary fibrosis
progressive interstitial fibrosis, variable associated inflammation, finger clubbing
what is sub pleural
between the pleura and body wall
describe the pathology of lungs during idiopathic pulmonary fibrosis
subpleural and basal fibrosis, terminal structure replaced by dilated spaces surrounded by fibrous walls, alveolar walls thickened
how does idiopathic pulmonary fibrosis affect gas exchange
impaired due to thickened alveolar walls
what is associated with late stage idiopathic pulmonary fibrosis
honeycombing
what are parts of the airway involved with chronic inflammatory disease
small airways, interstitium
describe inflammatory interstitial expansion and what it leads to
when tissue expanded by inflammatory tissue (not fibrosis). will eventually lead to fibrosis and scarring if inflammation persists
what is EAA
extrinsic allergic alveolitis
what causes inflammatory interstitial expansion
extrinsic allergic alveolitis
what is sarcoidosis
multi system granulomatous disorder of unknown cause
what immune response leads to granuloma formation
type 4 ( t cell mediated)
what are the other manifestations of sarcoidosis
uveitis, erythema nodosum, lymphadenopathy, hypercalcaemia (abnormal calcium metabolism)
what eventually happens to granulomas in interstitial lung disease
calcify
what connective tissue diseases affect the lungs
intersitial fibrosis, pleural effusions, rheumatoid nodules (can form in the lungs)
what is milder, idiopathic pleural fibrosis or interstitial fibrosis
interstitial
what is a differential diagnosis for rheumatoid nodules
TB
what is pneumoconiosis
group of occupational (restrictive) lung diseases caused by mineral dust exposure
name three forms of pneumoconiosis
asbestosis, coal workers lung, silicosis
what is the lungs reaction to inhalation of coal dust
fibrosis
what are the pathological features of coal miner lung
pigmentation, fibrosis, scarring and nodular scarring
what does pneumoconiosis dependant on
particle size, reactivity of particle, clearance of particle, host response
which shape of asbestos are most dangerous
straight
what results from the inhalation of asbestos
parietal pleural plaques, interstitial fibrosis (asbestosis), bronchial carcinoma, mesothelioma
